In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities.
Jean Y. Douet +11 more
doaj +1 more source
"Alternative" endocytic mechanisms exploited by pathogens: new avenues for therapeutic delivery? [PDF]
Some pathogens utilize unique routes to enter cells that may evade the intracellular barriers encountered by the typical clathrin-mediated endocytic pathway.
Medina-Kauwe, LK
core +1 more source
Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin [PDF]
Disease-related PrPSc [pathogenic PrP (prion protein)] is classically distinguished from its normal cellular precursor, PrPC(cellular PrP) by its detergent insolubility and partial resistance to proteolysis. Although molecular diagnosis of prion disease has historically relied upon detection of protease-resistant fragments of PrPSc using PK (proteinase
Cronier, Sabrina +6 more
openaire +4 more sources
Molecular Dynamics Studies on 3D Structures of the Hydrophobic Region PrP(109-136) [PDF]
Prion diseases caused by the conversion from a soluble normal cellular prion protein into insoluble abnormally folded infectious prions, are invariably fatal and highly infectious degenerative diseases that affect a wide variety of mammalian species. The
Zhang, Jiapu, Zhang, Yuanli
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Human Keratinocytes Express Cellular Prion-Related Proteinin Vitro and during Inflammatory Skin Diseases [PDF]
Prion diseases are transmissible spongiform encephalopathies of humans and animals characterized by the accumulation of a proteinase-resistant isoform of the cellular prion-related protein (PrPc) within the central nervous system. In the present report we demonstrate for the first time the presence of PrPc on squamous epithelia of normal and diseased ...
J, Pammer, W, Weninger, E, Tschachler
openaire +2 more sources
C. elegans Models to Study the Propagation of Prions and Prion-Like Proteins
A hallmark common to many age-related neurodegenerative diseases, such as Alzheimer’s disease (AD), Parkinson’s disease (PD), and amyotrophic lateral sclerosis (ALS), is that patients develop proteinaceous deposits in their central nervous system (CNS ...
Carl Alexander Sandhof +3 more
doaj +1 more source
Antihypertensive drug guanabenz is active in vivo against both yeast and mammalian prions. [PDF]
BackgroundPrion-based diseases are incurable transmissible neurodegenerative disorders affecting animals and humans.Methodology/principal findingsHere we report the discovery of the in vivo antiprion activity of Guanabenz (GA), an agonist of alpha2 ...
Déborah Tribouillard-Tanvier +9 more
doaj +1 more source
Computational Studies of the Structural Stability of Rabbit Prion Protein Compared to Human and Mouse Prion Proteins [PDF]
Prion diseases are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. The neurodegenerative diseases such as Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob diseases, Gerstmann-Str$\ddot{a}$ussler ...
Zhang, Jiapu
core +2 more sources
Prion degradation pathways: Potential for therapeutic intervention [PDF]
Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the misfolding of native cellular PrP(C) into the disease-associated form PrP(Sc) that accumulates in the brain as disease progresses. Although treatments have yet to be
Goold, R, McKinnon, C, Tabrizi, SJ
core +1 more source
How susceptible pigs are to infection with sheep prions is unknown. We show, through transmission experiments in transgenic mice expressing porcine prion protein (PrP), that the susceptibility of this mouse model to bovine spongiform encephalopathy (BSE)
Juan-Carlos Espinosa +8 more
doaj +1 more source

